Chiquet C, Lumbroso L, Denis P, Papo T, Durieu I, Lehoang P
Department of Ophthalmology, Edouard Herriot Hospital, Lyon, France.
Retina. 1999;19(4):309-13. doi: 10.1097/00006982-199907000-00007.
To report acute posterior multifocal placoid pigment epitheliopathy (APMPPE) in the course of systemic Wegener's granulomatosis.
A complete ophthalmologic evaluation was undertaken, including fluorescein and indocyanine green angiography.
Two patients diagnosed with Wegener's granulomatosis developed multiple white-yellow subretinal areas with smooth margins typical of APMPPE. Case 1 presented with bilateral choroidal involvement and a granulomatous anterior uveitis. In Case 2, placoid lesions were diagnosed 2 months after onset of systemic Wegener's granulomatosis. In both cases, fluorescein angiography showed an early hypofluorescence and a late staining of the lesions. Indocyanine green angiography revealed early and late hypofluorescence corresponding to the areas seen clinically. Wegener's granulomatosis was diagnosed histologically in both cases. After combined steroid and cyclophosphamide therapy, the lesions healed, and after a follow-up period of 1.5 and 4 years, respectively, funduscopic and angiographic examinations showed cicatricial lesions.
Acute posterior multifocal placoid pigment epitheliopathy is a rare manifestation of Wegener's granulomatosis and should be considered a possible systemic involvement of the disease. A prompt, thorough investigation should be undertaken for an underlying systemic disorder.
报告系统性韦格纳肉芽肿病程中出现的急性后极部多灶性扁平状色素上皮病变（APMPPE）。
进行了全面的眼科评估，包括荧光素和吲哚菁绿血管造影。
两名被诊断为韦格纳肉芽肿的患者出现了多个边界光滑的白色至黄色视网膜下区域，这是APMPPE的典型表现。病例1表现为双侧脉络膜受累及肉芽肿性前葡萄膜炎。病例2在系统性韦格纳肉芽肿发病2个月后诊断出扁平状病变。在这两个病例中，荧光素血管造影显示病变早期低荧光，晚期染色。吲哚菁绿血管造影显示与临床所见区域相对应的早期和晚期低荧光。两个病例均经组织学诊断为韦格纳肉芽肿。在联合使用类固醇和环磷酰胺治疗后，病变愈合，分别经过1.5年和4年的随访期后，眼底和血管造影检查显示为瘢痕性病变。
急性后极部多灶性扁平状色素上皮病变是韦格纳肉芽肿的一种罕见表现，应被视为该疾病可能的全身累及情况。对于潜在的全身性疾病应进行迅速、全面的调查。
